Life With a Dying Liver
One day, Don Dietz was a healthy 52-year-old on an eight-mile bike ride, and the next day, he was bleeding to death from an illness he'd never heard of.
It was December 1991, and Dietz had just flown in from Lubbock, Texas, where he was a professor of Spanish literature. Dietz's wife, Kathy, had already moved to Tempe, and Dietz was hoping to find work here as well.
He'd just had a complete physical examination at his university's medical school in September, and he felt healthy--until that morning when he couldn't raise himself out of bed. Kathy called an ambulance, and the emergency-room doctors found that he was bleeding profusely from his esophagus into his stomach, but they weren't sure why.
Dietz spent Christmas and New Year's in the hospital, and, without a good explanation, his gastroenterologist discharged him and told him that he would get better. He didn't.
He was wasting away, losing upper-body mass but getting thicker and thicker around the middle as his abdomen mysteriously filled with fluids.
"I looked like I was 94," he says, except that he seemed to have a great tan, even though he wasn't spending much time in the sun; he later realized that he was jaundiced.
He went from specialist to specialist, trying to find out why he looked and felt so bad. No one knew. One prominent gastroenterologist suggested he find a house near a hospital in case he hemorrhaged again.
Finally, the next April, when he was too weak to walk from his front door to the sidewalk--his wife had to roll him in a wheelchair--he checked into the Mayo Clinic in Scottsdale for a battery of tests. Within a week, doctors there found the problem.
Don Dietz had Hepatitis C, had had it for decades, and a liver biopsy confirmed that it had completely destroyed his liver. He had two choices: get a liver transplant or die.
"My wife and I cried all the way home from the Mayo Clinic," Dietz remembers. "I thought, 'Jesus Christ, you've got to be kidding!'"
Hepatitis C is a blood-borne disease that slowly strangles the liver. It's prevalent among intravenous drug users who share needles, and until accurate screening tests were perfected in 1992, it struck people who had received blood transfusions. Dietz fit neither of those categories, and he could only guess that he had contracted the disease in 1983 after being given a flu shot by a Catholic priest at the university in Ecuador where he was teaching. Perhaps the needle was dirty, he thought, but he couldn't be sure.
Dietz underwent a liver transplant in July 1992, and today he is healthy and active.
Hepatitis C is incurable, and the best Dietz can hope for is to buy another ten or 20 years of life before the new liver develops cirrhosis and hardens and ultimately fails as well.
Don Dietz had never heard of Hepatitis C, and chances are you haven't, either, even though the Centers for Disease Control estimate that about 3.9 million Americans have the disease, about three times the number of Americans infected with HIV, let alone who have full-blown AIDS.
It's not as sexy as AIDS, perhaps, less titillating. It's easier to consider AIDS and wag a scolding finger, knowing that we don't engage in the kinds of behaviors that get people in that kind of trouble. But the scary part about Hepatitis C is that it's much less discerning in whom it strikes.
One third of Hepatitis C patients got it from blood transfusions and one third from IV drug use, even if they never became drug addicts.
Marcia (not her real name) experimented with drugs when she was in graduate school in the late 1970s. She was living with a musician, and they would shoot up cocaine with friends. Marcia fled the drugs and the boyfriend, started a career as a schoolteacher in Phoenix, married and had children. Because she had never been addicted to drugs and had never overdosed--or had never even gotten sick--while using them, she thought she had gotten away with it. Nearly 20 years later, after confounding doctors with her frequent fevers and nausea, she learned that she hadn't.
"We often hear, 'If I had only known, I wouldn't have done this,'" says Lea Ann Nelson, a nurse at the Mayo Clinic who is the co-leader of a support group for people with liver disease.
Of course, that knowledge rarely stops anyone from smoking.
Many of the patients who present with Hepatitis C are in their 40s. Which doesn't mean that it's a baby-boomer disease so much as it's a disease that you can catch in your teens and 20s, the years when people are most prone to experiment with "bad" behaviors, and the symptoms don't show up until your 40s.
Most frightening is that the remaining third of Hepatitis C sufferers never misbehaved.
"There's about 35 percent of the people who don't have an identifiable risk factor," says Dr. David Douglas, a liver specialist at the Mayo Clinic.
"I've got one patient who's a teacher," adds Dr. Lawrence Koep, a surgeon at Samaritan Transplant Services. "She's married, monogamous, never did drugs, no transfusion. She has such a clean lifestyle that she could be a nun, and still she has it. We don't have a clue how she got it."
Experts can only theorize. Hepatitis C might be transmitted sexually--although many spouses of Hepatitis C patients never get it. It could probably be spread by sharing razors or nail clippers or even toothbrushes, certainly by accidental needle sticks or dirty acupuncture and ear-piercing needles.
Michelle Kiefhaber, a west-side Phoenix housewife who seems the picture of wholesomeness and health, thinks she contracted Hepatitis C from a tattoo she got 22 years ago, when she was 18. Now she's awaiting a liver transplant.
"This disease is worse than AIDS," says Jack Barritt, a molecular biologist and lab manager for National Genetics Institute, a California biotech company that processes most of the country's research tests that measure the virus levels for HIV and HCV (Hepatitis C Virus). After collecting a database of more than 40,000 Hepatitis C patients, he says, "AIDS is a more rapid disease. But this one fools the body into thinking you don't have an infection."
And then it leaves you in limbo.
"You hear so many stories," says Anthony Mercurio of Scottsdale. "Are you going to last two years? Three years? Are you going to be a candidate for liver transplant?"
Mercurio, 62, is a retired Army officer who was diagnosed with HCV in 1993. When the doctors first said "Hepatitis C," he countered with, "What's that?"
Now, as he enters yet another medical trial to see if something can slow his cirrhosis, he says, "I couldn't get those answers. And I understand now that I couldn't get those answers."
The liver may be the most important organ in the human body. It has been described as a chemical factory, but it's more like a combination toxic-waste and pharmaceutical conglomerate.
Not only does the liver remove from the blood the poisons we accidentally breathe in and the poisons--like alcohol and tobacco--that we deliberately ingest, but it also neutralizes the toxic by-products of living. Digestion creates large amounts of ammonia, for example, and if the ammonia weren't broken down into urea by the liver, it would find its way to our brains and cloud our judgment, a condition called encephalopathy.
But the liver also makes the proteins that make up our blood and stores fat and vitamins for later use. It converts glucose, the sugar our bodies use as fuel, into a stored form called glycogen. And, as if that weren't enough, the liver also regulates the amounts of cholesterol, testosterone and estrogen in our systems.
The term "hepatitis" does not refer to a type of virus, but merely to an end result, which is an inflammation of the liver.
Hepatitis can be caused by excessive alcohol and drug use, including over-the-counter analgesic drugs, or by a number of viruses. But most of the viruses are very different from each other. Hepatitis A comes from food or water that has been contaminated with infected feces. Hepatitis B is spread by shared needles and unprotected intercourse. There are hepatitis viruses labeled D, E and G, as well, and each has its own peculiarities.
HCV, like HIV, is a virus that affects RNA, one of the molecular components of all living cells. And like HIV, HCV is a mutating virus, which makes it hard to treat because it is ever adapting to whatever defenses the body's immune system sends at it. Hepatitis B, by contrast, does not mutate, and scientists have therefore been able to find a successful vaccine against it.
But as for the nature of the Hepatitis C virus, it is more closely related to St. Louis encephalitis, which affects the brain, and yellow fever, a mosquito-borne liver disease, than to HIV or the other hepatitis viruses.
The initial HCV infection may be mistaken for a case of the flu. And then it cooks slowly.
"This is not HIV, this is not Ebola," says liver specialist Dr. Alan Liebowitz. "This is something that smolders in the background. It's not a disease that kills people very quickly. It takes years and years to bump off a liver."
About 15 percent of Hepatitis C cases clear themselves out of the body. The remaining 85 percent become chronic, and the symptoms may arise in five or six years or as late as 20 years after infection.
For some patients, like Don Dietz, the symptoms come on suddenly. His internal bleeding is called "esophageal varices" and occurs when the blood flow is obstructed through the liver; the blood then tries to flow anywhere it can and ends up rupturing the tiny veins in the esophagus.
Other patients experience rashes and itching and chronic fatigue, and they go from doctor to doctor trying to figure out if they're sick or just crazy.
"Having Hepatitis C is like waking up with the worst case of flu you've ever had, every morning of your life," says Leslie Smith, 46, a Phoenix schoolteacher.
Sometimes the disease leads to liver cancer; more frequently it develops into cirrhosis in which the liver cells die and the liver hardens.
Doctors think that the drug interferon can slow down the Hepatitis C virus if it's administered early enough in the disease. But it does no good for patients whose livers are already cirrhotic. And even in those patients who have not yet reached cirrhosis, it only helps 50 percent of the cases--and half of those will relapse.
Even under the best of circumstances, interferon is the proverbial cure that is worse than the disease. Marcia, the schoolteacher who experimented with drugs, has been taking interferon for a year. And though it has kept her liver enzymes at near-normal levels, her journal of the first months taking the drug is a litany of migraines and nausea and days missed from work. This fall, Marcia will take part in a study at the Mayo Clinic that will combine interferon with an antiviral medicine called ribavirin. She remains ever hopeful that something will give her back her health.
But if the disease progresses to cirrhosis, the fluids that used to filter through the liver begin to back up in the abdomen. And as the levels of ammonia rise in the bloodstream because the liver can no longer convert it to urea, the patients can become increasingly addled.
Leslie Smith recalls standing in the produce section of a supermarket, suddenly unable to remember where she was, how she got there or how she could get out.
As a last-ditch effort, doctors can install a shunt in the liver to allow the fluids to pass through it without backing up. But this is a temporary measure, and when the liver fails completely, it must be replaced.
Assuming a liver can be found. Assuming the patient cooperates. Kevin Timothy, 42, a carpenter from Phoenix, got Hepatitis C from a blood transfusion in 1984. He'd also been an alcoholic--alcohol can accelerate the progression to cirrhosis--but claims to have controlled his problem. Until a year ago, he was a candidate for liver transplantation. But then he lapsed; he drank a beer in a moment of weakness and it turned up in a blood sample. Not wanting to waste a donor liver on an alcoholic, the doctors removed him from the liver-transplant waiting list.
"I know I'm dead," he says.
Before 1989, the only two hepatitis viruses that had been identified were A and B, and everything else fell under a catchall heading of non-A, non-B. Researchers now feel confident that the majority of those non-A and non-B cases were in fact caused by Hepatitis C.
But in 1989, the C virus was isolated and identified, and a year later, a test was found to detect the HCV antibodies in blood samples. In 1992, a more dependable antibody test was created, and more recently scientists have developed a test to measure the levels of the virus in the blood and identify the variant strains, or genomes, of the virus.
"I don't think it's a new disease," says Dr. Robert Clark, an infectious-disease specialist at Saint Joseph's Medical Center in Phoenix. "The technology got better, that's all. The disease is too newly described to know much about it.
"I have about 300 papers on Hepatitis C, but let me tell you, the stuff is rudimentary, and you can't answer simple questions like, what is the incidence?"
The CDC figure of 3.9 million is merely an estimate that amounts to roughly 1.5 percent of the general population.
Because Hepatitis C can now be excluded from the blood supply, one third of potential new infections should be prevented. And with increased education about needle-sharing, the segment that infects itself through drug use may shrink in the future.
While preferring to refer to the disease as a "major public health problem" and not an epidemic, Dr. Craig Shapiro, an epidemiologist at the CDC, says, "We don't have a good handle on the prevalence of the disease."
There's even less of a handle on the extent of infections in Arizona.
The Arizona Department of Health Services lists 52 cases of Hepatitis C in its 1995 Communicable Disease Summary, but that figure only comprises those few reported cases that fit narrow criteria for describing the disease.
There are certainly more cases out there. For example, United Blood Services, the blood bank that serves most of Arizona, excepting Tucson and southeastern parts of the state, has turned up 1,700 cases among would-be blood donors, 300 to 500 a year since the screening test became available in 1992.
As Dr. Thomas Wiltbank, medical director for United Blood Services in Arizona, points out, blood donors are among the healthiest subsets of the general population, and only make up about 10 percent. And so reason dictates that the figures are higher in the remaining 90 percent of Arizonans.
"The number of people with the virus is larger than we ever expected," Wiltbank says.
The same holds true for the nation as a whole.
"In the last two years, I've seen our calls increase dramatically," says Ari Maravel of the New Jersey-based American Liver Foundation. "We get about 150,000 calls a year, and about half of those are concerning Hepatitis C."
Susan Foster, a social worker for Samaritan Transplant Services who runs a liver-disease support group on behalf of the Arizona Liver Alliance, claims that over the past eight years, her Phoenix group has been increasingly dominated by Hepatitis C patients.
Physicians tend to regard the disease with doctorly detachment, because Hepatitis C, in their minds, is just a new name for an old disease. They still can't agree on some basic parameters, like whether it can be spread through sexual contact (it seems to spare the monogamous spouses of Hepatitis C patients, but afflicts people with multiple sex partners) and how frequently the virus clears up and how frequently it kills.
The National Institutes for Health have appropriated $8.4 million this year for Hepatitis C study, a paltry sum considering the American Liver Foundation's assertion that treatment cost $471 million in nonfederal hospital charges alone in the United States in 1994, but a start nonetheless.
Meanwhile, many Hepatitis C patients try to educate themselves. Because they can find precious little information in the general media, they surf the Internet for articles and chat lines and pure hearsay. They seek out natural remedies. They gather at monthly support groups for liver-disease patients to exchange information and case histories. There are five such groups in Arizona sponsored by the Arizona Liver Alliance: two in Phoenix (at the Mayo Clinic and at Samaritan Transplant Services), one in Flagstaff, and two in Tucson.
But many of their questions still go unanswered.
"The problem is that everyone is seeing it from a different perspective," says surgeon Lawrence Koep, "and probably the CDC statistics are going to give you the best idea of whether it's truly an epidemic or whether we're just discovering it now because of our tests.
"But I'll tell you," he continues, "the people I see now with it, it's bad news. There's no way of turning it around and no way of curing it."
Despite the surge in awareness of the disease, at least in the medical community, the liver-disease support groups are teeming with people who were misdiagnosed.
Their doctors at first did not add up the vague symptoms--the persistent colds, the skin rashes, the unexplainable aches and pains. They told their patients that they had lupus or ovarian cancer. And when they suspected liver trouble, they raised their eyebrows.
"Doctors won't tell you this," says Don Dietz, "but I have talked to fellow patients, and it happened to me. The first time there's an indication of elevated liver enzymes [a warning sign of liver disease], particularly for middle-aged men, they blame it on drinking."
"If the liver enzymes are elevated, usually what the doctors will say is, 'Oh, you must drink. Are you doing substances? What over-the-counter drugs are you taking?'" says Tamara Rounds, a social worker at the Mayo Clinic. "It's not 'til they rule out quite a few things that they realize that they need to screen for hepatitis."
When Mickey Mantle died after a liver transplant last year, the press hammered on his legendary boozing, when in fact what killed him was Hepatitis C, which led to liver cancer.
Marcia, the Phoenix schoolteacher who had experimented with drugs 20 years ago, was first diagnosed as having lupus. When her school district changed insurance companies and she subsequently changed doctors, her new doctor screened her for Hepatitis C and performed a liver biopsy to confirm his findings.
Leslie Smith's diagnosis was like one of those jokes that begin "I've got good news and bad news."
For years she'd suffered from chronic fatigue and body aches and skin rashes.
"Not the kind of itch that you can scratch," she says. "It's inside your body and it's trying to get out. No one could tell me what was wrong for years. I was treated as if I were psychologically impaired. I was on Valium and Librium. Oh my God, it was devastating to me."
Finally, Smith decided to find a new doctor, sat him down and read him the sum total of her symptoms, and he said, "You're really sick."
His tests confirmed that she had Hepatitis C.
At first Smith felt relieved because she finally knew that she wasn't crazy. Then she realized that she was dying.
She doesn't know how she got the disease in the first place.
"I can play what-if games," she says.
Smith is 46. When she was in college during the 1970s, she spent a lot of time trying to rescue some cousins who were shooting up drugs. They lived in squalor, and she would take it upon herself to clean up after them, sometimes picking up cotton swabs with blood on them. The cousins kicked the habit long ago, and from all appearances are perfectly healthy, she says.
But she's not doing so well herself. She's now on the waiting list for a liver transplant, but because she has a rare blood type, she does not expect to receive one soon.
When she still had enough strength to teach, she would instruct her students on what to do if she passed out in class or had some kind of accident.
"Don't touch me," she would tell them. "Get the nurse."
"I cut my finger the other night and I put the knife in the sink," she says. "And then I got to thinking, 'What if my mom doesn't wash that knife properly?'
"I feel like a leper.
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